[en] We report the successful management of a craniosynostosis repair in a child with severe Type I von Willebrand disease diagnosed during the preoperative assessment and treated by coagulation factor VIII and ristocetin cofactor. Collaboration among the anesthesiologist, the neurosurgeon, the clinical pathologist, and the pediatric hematologist is important for successful management.
Disciplines :
Surgery Life sciences: Multidisciplinary, general & others Anesthesia & intensive care
Author, co-author :
Maquoi, Isabelle ; Centre Hospitalier Universitaire de Liège - CHU > Anesthésie et réanimation
Bonhomme, Vincent ; Centre Hospitalier Universitaire de Liège - CHU > Anesthésie et réanimation
Duncan C, Richardson D, May P, Thiruchelvam J, Shong DC, Potter F, Grogan J, Caswell M. Reducing blood loss in synostosis surgery: the liverpool experience. J Craniofac Surg 2008;19:1424-30
Haas T, Fries D, Velik-Salchner C, Oswald E, Innerhofer P. Fibrinogen in craniosynostosis surgery. Anesth Analg 2008; 106:725-31 (Pubitemid 351301385)
Nichols WL, Hultin MB, James AH, Manco-Johnson MJ, Montgomery RR, Ortel TL, Rick ME, Sadler JE, Weinstein M, Yawn BP. von Willebrand disease (VWD): evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel report (USA). Haemophilia 2008;14:171-232 (Pubitemid 351343618)
Budde U. Diagnosis of von Willebrand disease subtypes: implications for treatment. Haemophilia 2008;14(suppl 5):27-38
Macfarlane DE, Stibbe J, Kirby EP, Zucker MB, Grant RA, McPherson J. A method for assaying von Willebrand factor (ristocetin cofactor). Thromb Diath Haemorrh 1975;34:306-8
Groner A. Pathogen safety of plasma-derived products-Haemate P/Humate-P. Haemophilia 2008;14(suppl 5):54-71
Mannucci PM. Treatment of von Willebrand's disease. N Engl J Med 2004;351:683-94
Kreuz W. von Willebrand's disease: from discovery to therapy-milestones in the last 25 years. Haemophilia 2008;14(suppl 5):1-2
Michiels JJ, Berneman ZN, van der PM, Schroyens W, Budde U, van Vliet HH. Bleeding prophylaxis for major surgery in patients with type 2 von Willebrand disease with an intermediate purity factor VIII-von Willebrand factor concentrate (Haemate-P). Blood Coagul Fibrinolysis 2004;15:323-30 (Pubitemid 38757060)
Thompson AR, Gill JC, Ewenstein BM, Mueller-Velten G, Schwartz BA. Successful treatment for patients with von Willebrand disease undergoing urgent surgery using factor VIII/VWF concentrate (Humate-P). Haemophilia 2004;10:42-51 (Pubitemid 38195624)
Mannucci PM, Bettega D, Cattaneo M. Patterns of development of tachyphylaxis in patients with haemophilia and von Willebrand disease after repeated doses of desmopressin (DDAVP). Br J Haematol 1992;82:87-93
Auerswald G, Kreuz W. Haemate P/Humate-P for the treatment of von Willebrand disease: considerations for use and clinical experience. Haemophilia 2008;14(suppl 5):39-46
Revel-Vilk S, Schmugge M, Carcao MD, Blanchette P, Rand ML, Blanchette VS. Desmopressin (DDAVP) responsiveness in children with von Willebrand disease. J Pediatr Hematol Oncol 2003;25:874-9 (Pubitemid 37413837)
Sutor AH. DDAVP is not a panacea for children with bleeding disorders. Br J Haematol 2000;108:217-27 (Pubitemid 30143632)
van Vliet HH, Kappers-Klunne MC, Leebeek FW, Michiels JJ. PFA-100 monitoring of von Willebrand factor (VWF) responses to desmopressin (DDAVP) and factor VIII/VWF concentrate substitution in von Willebrand disease type 1 and 2. Thromb Haemost 2008;100:462-8
Berntorp E. Prophylaxis in von Willebrand disease. Haemophilia 2008;14(suppl 5):47-53
Hans P, Collin V, Bonhomme V, Damas F, Born JD, Lamy M. Evaluation of acute normovolemic hemodilution for surgical repair of craniosynostosis. J Neurosurg Anesthesiol 2000;12:33-6 (Pubitemid 30040839)
Williams GD, Ellenbogen RG, Gruss JS. Abnormal coagulation during pediatric craniofacial surgery. Pediatr Neurosurg 2001; 35:5-12 (Pubitemid 32722313)
Kozek-Langenecker SA, Jungheinrich C, Sauermann W, Van der LP. The effects of hydroxyethyl starch 130/0.4 (6%) on blood loss and use of blood products in major surgery: a pooled analysis of randomized clinical trials. Anesth Analg 2008;107:382-90
Borel-Derlon A, Federici AB, Roussel-Robert V, Goudemand J, Lee CA, Scharrer I, Rothschild C, Berntorp E, Henriet C, Tellier Z, Bridey F, Mannucci PM. Treatment of severe von Willebrand disease with a high-purity von Willebrand factor concentrate (Wilfactin): a prospective study of 50 patients. J Thromb Haemost 2007;5:1115-24 (Pubitemid 46902109)
Walker JA, Dixon N, Gururangan S, Thornburg C. Perioperative factor IX replacement for surgical resection of a suprasellar astrocytoma in a child with severe haemophilia B. Haemophilia 2008;14:387-9 (Pubitemid 351338704)
Cermelj M, Negro F, Schijman E, Ferro AM, Acerenza M, Pollola J. Neurosurgical intervention in a haemophilic child with a subdural and intracerebral haematoma. Haemophilia 2004;10:405-7 (Pubitemid 39077043)
